Purpose
Report of a nationwide survey on lymphangioma conducted by the Korean Association of Pediatric Surgeons (KAPS) in 2019.
Methods
The authors reviewed and analyzed the clinical data of pediatric patients who started treatment for lymphangioma in hospitals of KAPS members from 2011 to 2013. Their follow-up data is also included in the study.
Results
A total of 532 patients with lymphangioma from 18 institutes were registered for the study. The results were discussed at the 35th annual meeting of KAPS, which was held in Gyeongju on June 13–14, 2019.
Conclusion
This study provides general information on lymphangioma and comprehensive treatment outcomes for this disease. The study is expected to be an important reference for improving pediatric surgeons’ understanding and treatment of lymphangioma.

Purpose
Gastroesophageal reflux disease (GERD) frequently affects patients with esophageal atresia (EA). GERD is often resistant to medical therapy, and anti-reflux surgery in the form of fundoplication is required. However, there are no generally accepted indications for fundoplication in EA patients with significant gastroesophageal reflux (GER). This study aimed to report the outcomes and review the clinical characteristics and treatment of GERD with Nissen fundoplication among EA patients.
Methods
The medical records of patients with EA repair who underwent Nissen fundoplication for GERD treatment from January 2005 to December 2018 were retrospectively reviewed. Twenty patients were included in this study.
Results
In total, 166 patients with EA underwent treatment during the study period. Among these, 20 (12%) patients underwent Nissen fundoplication. The median age at the time of fundoplication was 5.3 (range, 1.6–49.4) months, and the mean body weight was 6.3±2.4 kg. The indications for Nissen fundoplication were hiatal hernia in 18 (90%) patients, feeding intolerance in 17 (85%), recurrent aspiration pneumonia in 13 (65%), GER-related refractory anastomosis stenosis in seven (35%), and reflux-related acute life-threatening events in five (25%). The median follow-up duration after Nissen fundoplication was 5.7 (range, 2.4–10.7) years; two (10%) patients experienced surgery-related complications, 11 (55%) had recurrent hiatal hernia, and three (15%) required re-operation. Feeding intolerance improved (p<0.001), and the number of esophageal dilatations decreased following Nissen fundoplication (p=0.038). There was no mortality during the follow-up period.
Conclusion
Nissen fundoplication was required in patients with more complicated EA and was performed at a young age. Nissen fundoplication was relatively effective in controlling EA-associated GERD. However, Nissen fundoplication in patients with EA may be associated with a high rate of recurrent hiatal hernia. A careful follow-up is necessary because poor long-term outcomes are common for such patients.

Biliary atresia is a progressive sclerosing cholangiopathy of bile ducts. Most of the time it is an isolated anomaly but can present with syndromic forms. The biliary atresia splenic malformation (BASM) syndrome is associated with splenic anomalies, vascular anomalies, and visceral asymmetry along with biliary atresia. The surgical anatomy of BASM is distinctive and creates a challenge for the surgeons. We are describing a case of BASM with situs inversus and highlighting the approach, intraoperative anatomy, and surgical intricacies of Kasai portoenterostomy in such a situation with the review of pertinent literature.

Gallstone ileus occurs when there is a fistula between the gallbladder and the small intestine, by which the gallstone reaches the small bowel and thereby inducing bowel obstruction. This condition is very rare in the overall population, accounting for only 0.1–5%. It is also very rare in the pediatric population and in the patients who undergo Kasai operation as well. Worldwide, only two cases have been reported. Gallstone production may be facilitated under certain circumstances, and migrated stone induces ileus. Diagnosis is usually done by imaging studies such as abdominal ultrasonography and computed tomogram (CT). Surgical removal of stone is decisive treatment. The 22-year-old female patient with a history of Kasai operation at age of 1 month visited emergency department for abdominal pain. The most reasonable impression was the acute exacerbation of the chronic cholangitis, but it was improved soon after intravenous antibiotics treatment. At the same time, mechanical ileus was diagnosed and exacerbated during admission. CT was done to find out the cause. Small bowel obstruction by a round material was observed. By considering its clinical course and the imaging, the material was suspected to be gallstone. The patient underwent enterolithotomy under general anesthesia. She was fully recovered from gallstone ileus after stone removal. There has been no recurrence of ileus nor cholangitis. When a patient with a history of Kasai portoenterostomy complains mechanical ileus, the clinician need to allow for gallstone ileus referring patient’s history and medical condition which promotes to induce stone formation.

Catecholamine-producing tumors are rare, and the classical symptoms include maintained or paroxysmal hypertension, headaches, palpitations and sweating. However, its symptoms can vary greatly, which is why it has been called “the great masquerader.” We are reporting case of a patient with a left adrenal pheochromocytoma with multiple abdominal paragangliomas who presented with severe ischemic involvement of both the upper and lower limbs due to catecholamine induced vasospasm.

Infantile hemangiomas are the most common vascular tumors of infants and are characterized by abnormal proliferation of endothelial cells and aberrant blood vessel architecture. The drug Sirolimus has been explored as a new therapeutic option in the management of vascular anomalies that do not respond to conventional treatments. This report presents a case of refractory multiple infantile hemangiomatosis with brain involvement that was successfully treated with sirolimus.